Pathology of Bones Flashcards
What is the mutation found in achondroplasia?
1) FGF receptor 3 mutation (chromosome 4)
2) Inhibits cartilage proliferation
3) Autosomal dominant
Most common form of lethal dwarfism. What is the mutation associated with it?
1) Thanatophoric dwarfism
2) FGFR3 mutation
Diseases caused by the absence or mutation of type 1 collagen (alpha 1 and 2 chains)?
1) Osteogenesis Imperfecta
What are clinical features to type I osteogenesis imperfecta?
1) Blue sclera
2) Hearing impairment
3) Dental imperfections
4) Fractures in childhood
5) General decrease in bone
Rare genetic diseases that are characterized by reduced bone resoprtion and diffuse symmetric skeletal sclerosis; results in abnormally brittle bones What is the cell that is impaired?
1) Osteopetrosis
2) Osteoclasts are impaired
3) Osteoclast impairment is due to carbonic anhydrase II defect
Bones lack medullary canal, have persistence of primary spongiosa, and have erlenmeyer flask deformities; bones are sclerotic and thick, but are weak and brittle
Osteopetrosis
Is woven bone in adults normal?
No, abnormal
What are the three phases of Paget disease of the bone? What is the net result?
1) Initial osteolytic stage
2) Mixed osteoclastic-osteoblastic stage
3) Burnt out quiescent osteosclerotic stage
4) Gain of bone mass, but newly formed bone is disordered and structurally unsound
What is the hallmark for Paget disease of the bone?
1) Mosaic pattern of lamellar bone
What are complications of Paget disease of the bone?
1) High Output cardiac failure
2) Osteosarcoma
3) Osteoarthritis (due to bowing of the legs)
What are the findings of Osteitis fibrosa cystica (severe hyperparathyroidsim)?
1) Increased bone cell activity
2) Peritrabecular fibrosis
3) Cystic brown tumors
- Pigeon breast deformity
- Bowed legs
- Rachitic rosary
- Frontal bossing
Ricketsia
What skeletal changes can occur with renal osteodystrophy (five total)
1) Increased osteoclastic bone resoprtion (mimics osteitis fibrosa cystica)
2) Delayed matrix mineralization (mimics osteomalacia)
3) Growth retardation
4) Osteopororsis
5) Osteosclerosis
How does secondary parathyrodism occur in renal failure?
1) Renal failure results in hyperphosphatemia and decreased 1,25 dihydroxycholecalciferol
2) Increased phosphate and decreased vit. D results in increased release of PTH
3) PTH acts on osteoblasts which act on osteoclasts activating them
What is the worst subtype of osteoimperfecta? Which one can you live with?
1) Type II
2) Type I
What is the cause of osteopetrosis?
1) Impaired osteoclast activity due to mutation in carbonic anhydrase II
How do you differentiate osteoporosis from osteomalacia on radiograph?
1) Osteomalacia has trabeculae are surrounded by wide osteoid seams/osteoid matrix accumulation around trabeculae
2) Osteoporosis has even loss of mineralized and non-mineralized components of bone; trabecular thinning with fewer interconnections
What is the difference between senile and postmenopausal osteoporosis?
1) Senile = decreased activity of osteoblasts
2) Postmenopausal = increased activity of osteoclasts
How does hyperparathyroidism affect bone remodeling?
1) Increased PTH acts on osteoblasts to express RANKL
2) RANL binds to its receptor on osteoclasts activating them
3) Activated osteoclasts results in dissecting osteitis, cortical bone thinning, and brown tumors
What are three causes for osteonecrosis?
1) Idiopathic
2) Trauma
3) Corticosteroid administration
What organism are pt. at risk for osteomyelitis if they have sickle cell anema
Salmonella
What is the most common cause of osteomyelitis?
Staphylococcus aureus
Newly deposited bone forming sleeve of living tissue around the segment of devitalized infected bone
Involucrum
What are the radiographic findings of osteomyelitis?
1) Lytic focus of bone destruction surrounded by a zone of sclerosis
Describe Pott disease. What is its cause?
1) Myobacterium tuberculosis
2) Infection breaks through the intervertebral discs to involve multiple vertebrae and extend into the soft tissue forming abscesses
Dead piece of bone
Sequestreum